This Article  • Full text  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on ISI (81)  • Contact me when this article is cited  Related Content  • Related article  • Similar articles in this journal  Topic Collections  • Renal Diseases  • Renal Diseases, Other  • Dermatologic Disorders, Other  • Alert me on articles by topic

William W. Ting, MD, MBA; Mary Seabury Stone, MD; Kathi C. Madison, MD; Kevin Kurtz, MD

Arch Dermatol. 2003;139:903-906.

Background  There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic features. Soft tissue calcification is rare in patients with NFD and systemic involvement has not been reported.

Observations  We describe a patient with end-stage renal disease who developed diffuse indurated woody plaques consistent with NFD in association with soft tissue calcification with catastrophic sequelae. A deep excisional biopsy specimen from the patient revealed thickened collagen bundles in the reticular dermis, plump bipolar spindle cells, and increased mucin. Focally, there were zones of calcium deposition in dermal collagen without vessel calcification. Autopsy of the patient revealed extensive fibrosis and calcification of the diaphragm, psoas muscle, renal tubules, and rete testes. The patient died 11 months after developing NFD.

Conclusion  A subset of patients with NFD may have significant systemic involvement.

From the Departments of Dermatology (Drs Ting, Stone, and Madison) and Pathology (Drs Stone and Kurtz), University of Iowa College of Medicine, Iowa City. The authors have no relevant financial interest in this article.

RELATED ARTICLE

What Nephrogenic Fibrosing Dermopathy Might Be
Philip E. LeBoit
Arch Dermatol. 2003;139(7):928-930.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Incidence of Nephrogenic Systemic Fibrosis at Two Large Medical Centers
Prince et al.
Radiology 2008;248:807-816.
ABSTRACT | FULL TEXT  

Nephrogenic Systemic Fibrosis: A Report of 29 Cases
Shabana et al.
Am. J. Roentgenol. 2008;190:736-741.
ABSTRACT | FULL TEXT  

Case 6-2008 -- A 46-Year-Old Woman with Renal Failure and Stiffness of the Joints and Skin
Kay et al.
NEJM 2008;358:827-838.
FULL TEXT  

Nephrogenic systemic fibrosis and its association with gadolinium exposure during MRI
ISSA et al.
Cleveland Clinic Journal of Medicine 2008;75:95-111.
ABSTRACT | FULL TEXT  

Nephrogenic systemic fibrosis after exposure to gadolinium in patients with renal failure
Othersen et al.
Nephrol Dial Transplant 2007;22:3179-3185.
ABSTRACT | FULL TEXT  

Gadolinium-enhanced MR Imaging and Nephrogenic Systemic Fibrosis: Retrospective Study of a Renal Replacement Therapy Cohort
Collidge et al.
Radiology 2007;245:168-175.
ABSTRACT | FULL TEXT  

New Insights into Nephrogenic Systemic Fibrosis
Swaminathan and Shah
J. Am. Soc. Nephrol. 2007;18:2636-2643.
ABSTRACT | FULL TEXT  

Nephrogenic Systemic Fibrosis: Relationship to Gadolinium and Response to Photopheresis
Richmond et al.
Arch Dermatol 2007;143:1025-1030.
ABSTRACT | FULL TEXT  

Nephrogenic Fibrosing Dermopathy Associated With Exposure to Gadolinium-Containing Contrast Agents--St. Louis, Missouri, 2002-2006
JAMA 2007;297:1542-1544.
FULL TEXT  

Nephrogenic Systemic Fibrosis: A Mysterious Disease in Patients with Renal Failure--Role of Gadolinium-Based Contrast Media in Causation and the Beneficial Effect of Intravenous Sodium Thiosulfate
Yerram et al.
CJASN 2007;2:258-263.
ABSTRACT | FULL TEXT  

Nephrogenic Systemic Fibrosis: A Population Study Examining the Relationship of Disease Development to Gadolinium Exposure
Deo et al.
CJASN 2007;2:264-267.
ABSTRACT | FULL TEXT  

The case for ultraviolet light therapy in nephrogenic fibrosing dermopathy--report of two cases and review of the literature
Wahba et al.
Nephrol Dial Transplant 2007;22:631-636.
FULL TEXT  

Gadolinium - a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?
Grobner
Nephrol Dial Transplant 2006;21:1104-1108.
FULL TEXT  

Case 35-2004: Nephrogenic Fibrosing Dermopathy
Taylor et al.
NEJM 2005;352:1723-1724.
FULL TEXT  

Case 35-2004 - A 68-Year-Old Man with End-Stage Renal Disease and Thickening of the Skin
Moschella et al.
NEJM 2004;351:2219-2227.
FULL TEXT  

Nephrogenic fibrosing dermopathy in two patients with systemic lupus erythematosus
Obermoser et al.
Lupus 2004;13:609-612.
ABSTRACT  

What Nephrogenic Fibrosing Dermopathy Might Be
LeBoit
Arch Dermatol 2003;139:928-930.
FULL TEXT